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Title: Cardiovascular benefits of moderate exercise training in Marfan syndrome: Insights from an animal model.
Author: Mas-Stachurska, Aleksandra
Siegert, Anna M.
Batlle, Montserrat
Gorbenko del Blanco, Darya
Meirelles, Thayna
Rubies, Cira
Bonorino, Fabio
Serra-Peinado, Carla
Bijnens, Bart
Baudin, Julio
Sitges Carreño, Marta
Mont Girbau, Lluís
Guasch i Casany, Eduard
Egea Guri, Gustavo
Keywords: Malalties hereditàries
Aneurismes aòrtics
Models animals en la investigació
Genetic diseases
Aortic aneurysms
Animal models in research
Issue Date: 25-Sep-2017
Abstract: BACKGROUND: Marfan syndrome (MF) leads to aortic root dilatation and a predisposition to aortic dissection, mitral valve prolapse, and primary and secondary cardiomyopathy. Overall, regular physical exercise is recommended for a healthy lifestyle, but dynamic sports are strongly discouraged in MF patients. Nonetheless, evidence supporting this recommendation is lacking. Therefore, we studied the role of long-term dynamic exercise of moderate intensity on the MF cardiovascular phenotype. METHODS AND RESULTS: In a transgenic mouse model of MF (Fbn1C1039G/+), 4-month-old wild-type and MF mice were subjected to training on a treadmill for 5 months; sedentary littermates served as controls for each group. Aortic and cardiac remodeling was assessed by echocardiography and histology. The 4-month-old MF mice showed aortic root dilatation, elastic lamina rupture, and tunica media fibrosis, as well as cardiac hypertrophy, left ventricular fibrosis, and intramyocardial vessel remodeling. Over the 5-month experimental period, aortic root dilation rate was significantly greater in the sedentary MF group, compared with the wild-type group (∆mm, 0.27±0.07 versus 0.13±0.02, respectively). Exercise significantly blunted the aortic root dilation rate in MF mice compared with sedentary MF littermates (∆mm, 0.10±0.04 versus 0.27±0.07, respectively). However, these 2 groups were indistinguishable by aortic root stiffness, tunica media fibrosis, and elastic lamina ruptures. In MF mice, exercise also produced cardiac hypertrophy regression without changes in left ventricular fibrosis. CONCLUSIONS: Our results in a transgenic mouse model of MF indicate that moderate dynamic exercise mitigates the progression of the MF cardiovascular phenotype.
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It is part of: Journal Of The American Heart Association, 2017, vol. 6, num. 9, p. e006438
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ISSN: 2047-9980
Appears in Collections:Articles publicats en revistes (Medicina)
Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)

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